• Congenital abnormality of the trachea and main bronchi\
• Characterized by cystic dilatation of the tracheobronchial tree
  
  
• Pathologic findings

• Atrophy or absence of elastic fibers and thinning of muscle of the trachea and central bronchi
• Rapid change to normal caliber at the 4^th-5th order of bronchi

• More common in males

• Especially African Americans
• Only 5% of patients with the disease are female

• Occurs most often during the third and fourth decades of life
• Has an autosomal recessive type of inheritance
• Allows trachea and main bronchi to become markedly dilated on inspiration with narrowing or collapse on expiration
• Abnormal airway dynamics and pooling of secretions in broad outpouchings predispose to the development of

• Pulmonary fibrosis
• Emphysema
• Bronchiectasis
• Chronic pulmonary suppuration

• Clinical findings

• Recurrent pneumonia
• Dyspnea
• Cough
• Hoarseness
• Production of copious amounts of purulent sputum consistent with bronchiectasis

• Signs and symptom range from minimal disease with preservation of pulmonary function to progressive disease leading to respiratory failure and death
  
  
• Imaging Findings

• Tracheobronchomegaly (see below)
• Tracheal size increases with Valsalva and narrows with Muller maneuver
• Saclike outpouchings between tracheal cartilages
• Pulmonary fibrosis
• Bulla
• Bronchiectasis

• Has been associated with Ehlers-Danlos syndrome

 

 

 

• The diagnosis can be confirmed by computed tomography

 

 

Mounier-Kuhn Syndrome. Two axial CT images of the thorax demonstrate marked dilatation of the trachea (T)
and right (R) and left (L) main bronchi in this patient with Mounier-Kuhn syndrome. Notice the bronchiectasis
(red arrows and red circle) in which the bronchi are larger than their accompanying blood vessel
and there is tram=tracking of thickened bronchial walls seen in profile.
For the same photo without the arrows, click here